Bases moleculares de la enfermedad de Huntington: Papel del estrés oxidativo

Translated title of the contribution: The molecular bases of Huntington's disease: The role played by oxidative stress

Inmaculada Tasset, F. Sánchez, I. Túnez

Research output: Contribution to journalReview article

23 Citations (Scopus)

Abstract

Introduction. Huntington's disease is a neurodegenerative, autosomal dominant disease that mainly affects the basal ganglia. The disorder is caused by mutation in the gene encoding the huntingtin protein (Htt), producing intracellular aggregates. The adult form (chorea with dementia) is the most frequent. It is characterized by unpredictable, spasmodic, involuntary and constant movements, mostly associated with psychiatric and cognitive alterations. Development. The main characteristics of Huntington's disease are: neuronal loss, glyosis, and accumulations of mutated Htt, all associated with different underlying channels in the pathogenesis of the disease, such as: excitotoxicity, energy deficit (ATP depletion), reduction in the synthesis and release of neurotrophic factors (BDNF and GDNF), and oxidative stress. Oxidative stress is involved in the pathogenesis of various neurodegenerative diseases, including Huntington's disease, and numerous studies have shown the existence of oxidative damage in the plasma and tissue of patients suffering from this disease. Conclusions. Oxidative stress in patients with Huntington's disease may be used as an evolutionary-prognostic marker of both the disease and therapeutic effectiveness, as well as an interesting field of research for the development of new therapeutic strategies.

Original languageSpanish
Pages (from-to)424-429
Number of pages6
JournalRevista de Neurologia
Volume49
Issue number8
StatePublished - Oct 15 2009
Externally publishedYes

Fingerprint

Huntington Disease
Oxidative Stress
Glial Cell Line-Derived Neurotrophic Factor
Chorea
Brain-Derived Neurotrophic Factor
Dyskinesias
Nerve Growth Factors
Basal Ganglia
Neurodegenerative Diseases
Psychiatry
Dementia
Adenosine Triphosphate
Mutation
Therapeutics
Research
Genes
Huntingtin Protein

Keywords

  • Cellular death
  • Huntington's disease
  • Oxidative stress
  • Reactive oxygen species

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Bases moleculares de la enfermedad de Huntington : Papel del estrés oxidativo. / Tasset, Inmaculada; Sánchez, F.; Túnez, I.

In: Revista de Neurologia, Vol. 49, No. 8, 15.10.2009, p. 424-429.

Research output: Contribution to journalReview article

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